Search

Background Telangiectasias of the lower extremities are very common. There are no blinded, randomized, controlled clinical trials comparing laser modalities with the gold standard sclerotherapy, while the few available studies encompass small patients cohorts. Objective This prospective, randomized, open-label trial compares the efficacy of sclerotherapy with polidocanol vs. long-pulsed neodymium-doped yttrium aluminium garnet (Nd: YAG) laser in the treatment of leg telangiectasias. Patients and methods Fifty-six female patients with primary leg telangiectasias and reticular veins (C1A or SEp AS1 PN) were included in the study. One leg was randomly assigned to get treatment with the multiple synchronized long-pulsed Nd: YAG laser, while the other received foam sclerotherapy with polidocanol 0.5%. The patients were treated in two sessions at intervals of 6 weeks. The patients were evaluated by the handling physician after 6 weeks and 6 months. Two investigators assessed blindly at the end of the study the photographs for clearing of the vessels using a six-point scale from 1 (no change) to 6 (100% cleared). Patients reported about pain sensation and outcome satisfaction. Results According to the handling dermatologist, at the last follow-up, there was an improvement of 30-40% with a median of 3 ( IQR 2) and a good improvement of 50-70% with a median of 4 ( IQR 2) after laser treatment and sclerotherapy respectively. In contrast, according to the blinded investigators, there was a median of 5 ( IQR 1) with a very good improvement of >70% after both therapies. Improvement was achieved more quickly by sclerotherapy, although at the last follow-up visit there was no difference in clearance between the two groups as assessed by the blinded experts ( P-value 0.84). The degree of patient's satisfaction was very good and similar with both therapeutic approaches. There was a significant difference ( P-value 0.003) regarding pain perception between the types of therapy. Laser was felt more painful than sclerotherapy. Conclusion Telangiectasias of the lower extremities can be successfully treated with either synchronized long-pulsed Nd: YAG laser or sclerotherapy. The 1064-nm long-pulsed Nd: YAG laser is associated with more pain and is suitable especially in case of needle phobia, allergy to sclerosants and in presence of small veins with telangiectatic matting, while sclerotherapy can also treat the feeder veins. [ABSTRACT FROM AUTHOR]

Background: Bullous pemphigoid (BP) is the most common autoimmune blistering skin disease and is associated with an increased mortality. The end points of our study were to evaluate the mortality rate in a retrospective cohort of BP patients followed up to 5 years after the diagnosis and to determine prognostics factors. Methods: All new cases of BP diagnosed between 1990 and 2003 in the University Hospital of Geneva were retrospectively collected. 60 patients were included, 47 (88.6%) of whom were treated with a combination of corticosteroids and chlorambucil. Results: The 1-year, 2-year and 5-year probabilities of death were 26.7, 37.1 and 60.8%, respectively. Old age, dementia and use of chlorambucil at initial doses of 6 mg/day, but not at lower doses, were associated with poor prognosis in multivariate analysis. Conclusion: Our study confirms that BP is associated with a high mortality. The observed mortality rates are however higher than those of previous studies, which is probably related to the inclusion of more debilitated patients. Copyright © 2012 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Background Prospective systematic analyses of the clinical presentation of bullous pemphigoid (BP) are lacking. Little is known about the time required for its diagnosis. Knowledge of the disease spectrum is important for diagnosis, management and inclusion of patients in therapeutic trials. Objectives The primary aims of the study were: (i) to characterize the clinical features of BP at time of diagnosis; and (ii) to assess the diagnostic delay in BP and its impact on prognosis Methods All new cases of BP diagnosed in Switzerland between 1 January 2001 and 31 December 2002 were prospectively registered by means of a standardized data collection form. Results One hundred-seventeen patients with BP were included in the study. 97cases (82.9%) had typical features with vesicles, blisters and/or erosions at time of diagnosis, while in the remaining cases (17.1%) only excoriations, eczematous and/or urticarial infiltrated lesions were observed. Head/neck as well as palmo-plantar involvement were found in up to 20% of patients, while mucosal lesions were present in 14.5% of the cases. Diagnosis was made after a mean of 6.1 months after the first symptoms. In patients, in whom the diagnostic delay was 4 months or more (defined as late diagnosis group), lesions were more often limited to one body area. The type of lesions did not affect the diagnostic delay. Diagnosis was made more rapidly in patients with limb involvement compared to those without. The calculated mortality rate in the early and late diagnosis group was 18.9% and 17.9%, respectively, without significant difference. Conclusion BP often presents with bullous lesions at time of diagnosis after a mean diagnostic delay of 6 months. Nevertheless, up to 20% of patients lack obvious blistering and postbullous erosions, mimicking thus a variety of inflammatory dermatoses. Localized disease is associated with an increased diagnostic delay, which has however no impact on prognosis. [ABSTRACT FROM AUTHOR]

Summary Background Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease of the skin associated with IgG autoantibodies to BP180 and BP230, while mucous membrane pemphigoid (MMP) comprises a heterogeneous group of autoimmune blistering diseases characterized by a predominant mucous membrane involvement and scarring tendency associated with an autoantibody response to various autoantigens, including BP180. While the pathogenicity of IgG autoantibodies to BP180 has been demonstrated in BP, the role of IgE autoantibodies in mediating tissue damage in BP and MMP is unclear. Objectives To assess the presence of tissue-bound IgE in patients with BP and MMP, and their correlation with distinct clinical features. Methods In this retrospective study, we assessed the presence of IgE deposits as detected by direct immunofluorescence microscopy of skin biopsy specimens obtained from 44 and 13 patients with a new diagnosis of BP and MMP, respectively. Distinct clinical features at time of diagnosis, such as itch, urticarial papules and plaques and eczematous lesions, were noted. Results In 18 of 44 (41%) patients with BP linear deposits of IgE of variable intensity were detectable along the dermoepidermal junction. In 14 (32%) of the cases, IgE deposits were found concomitantly with IgG and C3. In two (5%) patients, diagnosis of BP was based on the isolated detection of IgE together with consistent clinicopathological features. Nine of 13 (69%) patients with MMP also exhibited linear IgE deposits, including one case with isolated linear IgE deposits. Patients with BP with tissue-bound IgE deposits had clinically significant urticarial papules and plaques when compared with patients with BP without IgE deposits. Conclusions Our findings indicate that demonstration of tissue-bound IgE deposits provides an additional useful criterion for diagnosis of BP and MMP in some patients. Prospective studies are needed to better correlate the presence of tissue-bound and circulating IgE autoantibodies and their specificity with distinct clinical features and course of BP and MMP. [ABSTRACT FROM AUTHOR]

Summary [ABSTRACT FROM AUTHOR]

Background. Pemphigus herpetiformis (PH) is a rare dapsone-responsive variant of pemphigus, characterized by annular and vesiculopustular cutaneous lesions. Most PH serum samples contain autoantibodies against desmoglein (Dsg)1, but not Dsg3, and the presence of the latter is almost invariably associated with mucosal involvement, as predicted based on the ‘Dsg compensation theory’. Methods. We describe a patient with features characteristic of PH with histologically eosinophilic spongiosis who repeatedly tested positive for anti-Dsg3 but not anti-Dsg1 autoantibodies by ELISA. To investigate whether the peculiar clinical phenotype was due to a distinct immunological profile, the patient’s serum was tested by ELISA and immunoblotting using recombinant forms of Dsg3. Results. Serum samples were found to have low and high reactivity against the EC1 and the EC4 domains of Dsg3, respectively, whereas the autoantibodies belonged predominantly to the IgG1 and IgG4 subclasses. The overall immunological profile was typical of pemphigus vulgaris. The patient finally developed isolated oral erosions 22 months after initial presentation, without significant changes in the autoantibody profile and of the targeted antigenic sites. Conclusions. Our patient presented features characteristic of PH. Although circulating anti-Dsg3 antibodies were present, the patient had only cutaneous involvement for a long period. Our findings indicate that the proposed Dsg compensation theory cannot always explain the clinical phenotype, changes in which may occur without apparent modification of the autoantibody profile and antibody specificity. Hence, additional factors, such as Fcγ-dependent neutrophil activation, may critically affect the clinical presentation of pemphigus. [ABSTRACT FROM AUTHOR]

Background Bullous pemphigoid (BP), pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune bullous diseases characterized by the presence of tissue-bound and circulating autoantibodies directed against disease-specific target antigens of the skin. Although rare, these diseases run a chronic course and are associated with significant morbidity and mortality. There are few prospective data on gender- and age-specific incidence of these disorders. Objectives Our aims were: (i) to evaluate the incidence of BP and PV/PF in Swiss patients, as the primary endpoint; and (ii) to assess the profile of the patients, particularly for comorbidities and medications, as the secondary endpoint. Methods The protocol of the study was distributed to all dermatology clinics, immunopathology laboratories and practising dermatologists in Switzerland. All newly diagnosed cases of BP and pemphigus occurring between 1 January 2001 and 31 December 2002 were collected. In total, 168 patients (73 men and 95 women) with these autoimmune bullous diseases, with a diagnosis based on clinical, histological and immunopathological criteria, were finally included. Results BP showed a mean incidence of 12·1 new cases per million people per year. Its incidence increased significantly after the age of 70 years, with a maximal value after the age of 90 years. The female/male ratio was 1·3. The age-standardized incidence of BP using the European population as reference was, however, lower, with 6·8 new cases per million people per year, reflecting the ageing of the Swiss population. In contrast, both PV and PF were less frequent. Their combined mean incidence was 0·6 new cases per million people per year. Conclusions This is the first comprehensive prospective study analysing the incidence of autoimmune bullous diseases in an entire country. Our patient cohort is large enough to establish BP as the most frequent autoimmune bullous disease. Its incidence rate appears higher compared with other previous studies, most likely because of the demographic characteristics of the Swiss population. Nevertheless, based on its potentially misleading presentations, it is possible that the real incidence rate of BP is still underestimated. Based on its significant incidence in the elderly population, BP should deserve more public health concern. [ABSTRACT FROM AUTHOR]

Editor Since the first descriptions by Strutton I et al i .,1 neutrophilic dermatosis of the dorsal hands (NDDH) has now been recognized as a distinctive localized form of either pustular Sweet syndrome (SS) or bullous or ulcerative pyoderma gangrenosum (PG).2,3 Although the lesions are characteristically located on the dorsa of the hands and the fingers, additional locations may be affected.2-5 NDDH shows a rich inflammatory cell infiltrate mainly of neutrophils and abundant nuclear dust with variable sign of leucocytoclastic vasculitis.1-5 The clinical features, which pose a diagnostic challenge, are frequently misdiagnosed as infection, resulting in inappropriate antibiotics use or surgical procedures.1-5 The diagnosis of NDDH warrants an evaluation for occult malignancy, such as myelodysplasia, and systemic inflammatory diseases.1-5 Here, we describe a case of NDDH triggered by mechanical trauma. Light microscopy studies showed an oedema in the upper dermis with an inflammatory infiltrate composed of neutrophils. [Extracted from the article]

Multiple miliary osteoma cutis of the face represents a rare and frequently unrecognized complication of chronic inflammatory acne. Their differentiation from microcomedones and macrocomedones may be challenging. The case of a 46-year-old Asian woman who suffered from chronic inflammatory acne is described. She had multiple papular lesions of the cheeks that did not respond to various topical and systemic therapies including oral isotretinoin. Light microscopy studies as well as ultrasound and computed tomography (CT) scan investigations demonstrated the presence of multiple osteoma cutis. Needle microincisions followed by mechanical extirpation of the bony formation resulted in a considerable cosmetic improvement of her skin disease. Knowledge of this rare complication of acne is mandatory, as its treatment is different from that of retentional and inflammatory acne and frequently relies on surgical modalities. Our novel technique consisting of needle microincisions with curettage of the lesions is simple and safe, leading to good cosmetic results. [ABSTRACT FROM AUTHOR]